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Case report
Granulomatosis with polyangiitis presenting as pancreatic disease
  1. Jessie Jia Tao1,
  2. Arnav Agarwal1,
  3. Ari Benjamin Cuperfain2 and
  4. Christian Pagnoux1,3
  1. 1Department of Medicine, University of Toronto, Toronto, Ontario, Canada
  2. 2Department of Psychiatry, University of Toronto, Toronto, Ontario, Canada
  3. 3Division of Rheumatology, University of Toronto, Toronto, Ontario, Canada
  1. Correspondence to Dr Christian Pagnoux; Christian.pagnoux{at}sinaihealth.ca

Abstract

Granulomatosis with polyangiitis (GPA) is a rare necrotising small vessel vasculitis typically associated with oronasal, pulmonary and renal manifestations. Pancreatic disease is an exceedingly rare initial presentation and is associated with delayed diagnosis and rapid progression. We discuss a 66-year-old woman presenting with epigastric pain, elevated lipase and radiographic evidence of focal pancreatitis. She had no relevant medical history and no lithiasis seen on imaging. Pertinent findings include strawberry gingivitis, positive proteinase-antineutrophil cytoplasm antibody (98% specificity) and focal nodular parenchymal lung lesions on CT chest—all of which are consistent with a diagnosis of GPA. She was promptly started on high-dose steroids which resulted in significant clinical and biochemical improvement. Cyclophosphamide was added once biopsy confirmed the absence of malignancy. In order to optimise the clinical outcomes of GPA, physicians must keep a wide differential and high index of suspicion in the setting of unexplained pancreatitis with systemic features.

  • rheumatology
  • vasculitis
  • gastroenterology
  • pancreatitis

https://doi.org/10.1136/bcr-2020-241033

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    Footnotes

    • Twitter @jessietao_, @ArnavAgarwalMD, @AriCuperfain

    • Contributors All coauthors contributed to the content of this case report, provided final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. JJT, AA and CP conceived and designed the project. JJT, AA and ABC were involved in data acquisition and interpretation of data. JJT prepared the first draft of the manuscript. AA, ABC and CP provided critical revisions to the manuscript. Patient consent was obtained following review of the final full-text manuscript. Upon resubmission, JJT prepared the revision and all authors approved the final version of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.